Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifty-five patients with cytomegalovirus (CMV)-associated neonatal hepatitis (NH) were followed for 12 to 90 months. Six patients (10.9%) died from either a fulminant course or a chronic liver disease. Among the remaining 49 patients, whose liver function was completely recovered, there were eight with retardation of developmental or growth status, and two with hearing impairment. Overall, 20.4% of the survivors suffered from a long-term impact. The unfavorable outcome was related to several clinical and pathological parameters. These included persistence of clay-colored stool, presence of splenomegaly, ascites or anemia, high peak total and direct bilirubin, low nadir albumin levels, diffuse giant cell transformation and cirrhosis of the liver. The seropositivity of CMV infection did not significantly correlate with the outcome.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Cytomegalovirus-associated neonatal hepatitis. 133 53

The maturing reticulocyte degrades ribosomal RNA to constituent ribonucleoside phosphates. Guanosine ribonucleotides are retained only in small amounts and pyrimidine ribonucleotides only in trace quantities. In the mature erythrocyte more than 97% of total nucleotides are the interconvertible adenosine mono-, di-, and triphosphates. High energy ATP fuels most of the reactions required to sustain viability. Unable to synthesize adenosine phosphates from small precursor molecules, the red cell relies on certain salvage pathways to replenish its losses from the adenosine phosphate pool. The most important of these involve adenosine. Adenylate kinase deficiency, when severe, is associated with nonspherocytic hemolytic anemia. A genetically-determined deficiency of pyrimidine 5'-nucleotidase prevents the normal dephosphorylation of pyrimidine ribonucleotides, and hence is characterized by the unique accumulation of pyrimidine phosphates intracellularly. Other features are chronic hemolytic anemia, splenomegaly, and a profound increase in basophilic stippling on the stained blood film. The syndrome is transmitted as an autosomal recessive disorder. A similar syndrome is found in severe lead poisoning as a consequence of nucleotidase inhibition by lead. An inherited, dominantly transmitted hemolytic anemia associated with low red cell ATP and a 45-70 fold increase in the enzymatic activity of adenosine deaminase has also been documented. The undefined molecular lesion appears to involve overproduction of an entirely normal enzyme protein. Severe deficiency of either of two sequential enzymes of purine metabolism, adenosine deaminase anemia, but by excessive accumulations of deoxyribonucleotides within red cells and lymphocytes. The clinical counterpart of each is a severe immunodeficiency state secondary to lymphopenia and lymphocyte dysfunction. Certain other rare clinical syndromes involving disturbed nucleotide metabolism also are detectable by red cell assay procedures.
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PMID:Erythrocyte disorders of purine and pyrimidine metabolism. 625 19

Friend leukemia is caused by Friend leukemia virus (FLV) in susceptible mice. The malignant cells are of erythrocyte and lymphoid cell origin. Spleen enlargement could be detected within two weeks of FLV inoculation. The diseased mice usually die in the third or fourth month. Specific antisera were prepared from chimpanzee or rabbit by injection of FLV or FLV gp 71 in Freund's adjuvant. In the study of immunotherapy, DBA/2J mice were used. Normally, DBA/2J mice have an average spleen weight of 100 mg. After inoculation with FLV, the spleen enlarged to around 1,000 mg. Chimpanzee anti-FLV and chimpanzee anti-FLV gp 71 antisera were effective in treatment of FLV infection. The spleen enlargement could be recovered to normal range. The survival also was remarkably prolonged. Rabbit anti-FLV gp 71 antiserum was partially effective in retardation of spleen enlargement after FLV inoculation. On the contrary, 2 lots of rabbits anti-FLV antisera were found to accelerate spleen enlargement. Possibility of the presence of enhancement factor is considered. Therefore, different reactions in different species of animal to FLV vaccination are evident.
Zhonghua Min Guo Wei Sheng Wu Ji Mian Yi Xue Za Zhi 1980 Mar
PMID:[Immunotherapy of Friend leukemia with antiserum (author's transl)]. 744 27